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芝加哥某儿童医院1976～1983年间有81个儿童死于肝衰,其中除5人属继发性肝衰外(3例先天性心脏病,2例中枢神经系统感染),余76人为原发性肝衰,包括胆道闭锁20例,代谢性疾病22例,Reye综合征7例,感染15例,胆汁淤滞12例,杂症5例。因肝脏疾病而死亡的人数占该院同期死亡总数的6.6%。有8人作肝移植术,存活6人。死亡年龄以小婴儿为多,占近半数,包括胆道闭锁病人全部的2/3和代谢性疾病如酪氨酸代谢紊乱症,囊性纤维性变,α_1-抗胰蛋白酶缺乏,Wilson病,Zellwegeo综合征及染色体异常;其次为家族性胆汁淤滞综合症。平均死亡年龄为14个月。Reye综合征死亡年龄在6～11岁。因感染死亡的年龄范围较
A child hospital in Chicago from 1976 to 1983, 81 children died of liver failure, of whom 5 were secondary to liver failure (3 cases of congenital heart disease, 2 cases of central nervous system infection), more than 76 were primary Liver failure, including biliary atresia in 20 cases, metabolic diseases in 22 cases, Reye syndrome in 7 cases, infection in 15 cases, 12 cases of cholestasis, 5 cases of miscellaneous diseases. The number of deaths due to liver disease accounted for 6.6% of the hospital total deaths over the same period. There are 8 people for liver transplantation, survival of 6 people. The death ages are as many as a baby, accounting for nearly two-thirds of all biliary atresia patients and metabolic disorders such as tyrosine metabolic disorders, cystic fibrosis, alpha 1-antitrypsin deficiency, Wilson’s disease, Zellwegeo Syndrome and chromosomal abnormalities; followed by familial cholestasis syndrome. The average age of death is 14 months. Reye syndrome death age at 6 to 11 years old. The age range of deaths due to infection